What Is Iridocorneal Endothelial Syndrome (ICE)?

Iridocorneal endothelial syndrome (ICE) is a rare eye condition whose three main features include: 

• Swelling of the cornea, which is the clear, front part of the eye;
• Changes in the iris, which is the colored part of the eye; and
• A form of glaucoma.

ICE is actually a group of conditions related to changes in corneal cells and the iris. The syndrome almost always involves cells moving from the cornea to the iris. Loss of cells from the cornea can cause corneal swelling, and the iris and pupil can become distorted. Also, when the corneal cells move, they can block fluid from draining properly through the eye’s microscopic drainage channels. This blockage causes pressure in the eye to build, leading to glaucoma.

Who is at Risk for Iridocorneal Endothelial Syndrome (ICE)?

Women are diagnosed with iridocorneal endothelial syndrome more often than men. The syndrome is usually diagnosed in midlife. Typically, ICE affects only one eye.

Causes

Though the causes of ICE are not fully known, some physicians believe the syndrome may be triggered by a virus, such as herpes simplex, that then causes the cornea to swell.

Symptoms

People with iridocorneal endothelial syndrome may have pain or blurry vision in one eye or notice changes in the iris or pupil. An exam by an ophthalmologist will generally show changes in the iris, swelling of the cornea and increased pressure in the eye (glaucoma).

Diagnosis

An ophthalmologist will perform a complete eye exam and some other basic tests to diagnose iridocorneal endothelial syndrome.

Treatment

There is no way to stop the progression of ICE, but treatment is usually focused on managing the glaucoma, either through medication or possibly surgery, to help reduce pressure in the eye. Medication might also be prescribed to help reduce corneal swelling. In some cases, a cornea transplant may be necessary.